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Las lipodistrofias son trastornos poco frecuentes que afectan al tejido adiposo, principalmente al metabólicamente activo, tanto subcutáneo como visceral, y que se caracterizan por la desaparición del mismo. La pérdida de tejido adiposo puede afectar a la práctica totalidad del mismo –como en las lipodistrofias generalizadas– o sólo a una parte –como en las lipodistrofias parciales y localizadas–. Las lipodistrofias pueden ser genéticas o adquiridas, y estas últimas suelen tener una base inmunológica. Con relativa frecuencia y generalmente debido a la extensa pérdida de tejido adiposo, las lipodistrofias se asocian a una serie de complicaciones metabólicas como son la resistencia a la insulina, la hipertrigliceridemia, el descenso del colesterol HDL y la hipoleptinemia. También es frecuente la asociación con la acantosis nigricans, la esteatosis hepática y el síndrome del ovario poliquístico. Esta revisión tiene como objetivo describir las características clínicas y los criterios diagnósticos de los distintos tipos de lipodistrofias y profundizar en las bases moleculares de las mismas.
Palabras clave:
Tejido adiposo
Resistencia a la insulina
Hipertrigliceridemia
Laminina A/C
Gen LMNA
Lipodystrophies are rare diseases which affect adipose tissue, usually metabolically active tissue, both subcutaneous and visceral, and which are characterized by fat loss. This loss of fat can affect almost the entire body, as in generalized lipodystrophies, or only part of the body, as in partial and localized lipodystrophies. The causes of lipodystrophies can be genetic or acquired, and the latter usually have an immunological basis.
Lipodystrophies are frequently associated with a series of metabolic complications such as insulin resistance, hypertriglyceridemia, low HDLcholesterol and hypoleptinemia, which are usually related to the extent of fat loss. Acanthosis nigricans, hepatic steatosis and polycystic ovaries are also frequently associated. The aim of this review is to describe the clinical features and diagnostic criteria of the different types of lipodystrophies, as well as to provide further information on their molecular bases.
Key words:
Adipose tissue
Insulin resistance
Hypertriglyceridemia
Lamin A/C
LMNA gene
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